Jacobse Justin, Sijpkens Yvo W J, van 't Wout Jan W, Peters Elke E M, Vlasveld L Tom
Department of Internal Medicine, HMC Bronovo, The Hague, Netherlands.
Department of Pathology, HMC Bronovo, The Hague, Netherlands.
J Hematol. 2018 Dec;7(4):158-162. doi: 10.14740/jh461w. Epub 2018 Nov 22.
There is a clear association between myelodysplastic syndrome (MDS)/chronic myelomonocytic leukemia (CMML) and autoimmune manifestations such as vasculitis. It is not clear if autoimmune manifestations in myelodysplastic syndrome are a cause or consequence. We describe two patients with polyarteritis nodosa and large vessel vasculitis, as presenting symptom of a myelodysplastic syndrome with excess blasts type 2 and chronic myelomonocytic leukemia respectively. Immunosuppressive treatment resulted in amelioration of the vasculitis with improvement of the myelodysplastic features in the first patient and rapid evolution to acute myeloid leukemia in the other patient. The association between MDS/CMML and autoimmune manifestations, such as vasculitis, emphasizes the role of autoimmunity in the clinical features and even pathogenesis of MDS/CMML.
骨髓增生异常综合征（MDS）/慢性粒单核细胞白血病（CMML）与自身免疫表现（如血管炎）之间存在明确关联。目前尚不清楚骨髓增生异常综合征中的自身免疫表现是病因还是结果。我们描述了两名分别患有结节性多动脉炎和大血管血管炎的患者，它们分别是2型原始细胞过多型骨髓增生异常综合征和慢性粒单核细胞白血病的首发症状。免疫抑制治疗使第一名患者的血管炎得到改善，骨髓增生异常特征也有所改善，而另一名患者则迅速演变为急性髓系白血病。MDS/CMML与自身免疫表现（如血管炎）之间的关联强调了自身免疫在MDS/CMML临床特征甚至发病机制中的作用。
